Outer Retina Analysis by Optical Coherence Tomography in Cone-Rod Dystrophy Patients

Lima, Luiz H.; Sallum, Juliana Maria Ferraz; Spaide, Richard F.

doi:10.1097/iae.0b013e31829234e6

Cited by 40 publications

(25 citation statements)

References 15 publications

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“…ERG is a more sensitive technique and can be used to diagnose CD earlier than is possible using current diag nostic techniques (1) . A variety of mechanisms, such as defective outer segment morphogenesis, protein transport along the cilium, phototransduction, or cellular interaction, have been suggested as being responsible for cone dysfunction (2) . The retinal pathology of CD occurs mainly between the photoreceptor outer segment and retinal pigment epithelium (RPE) layer (1) .…”

Section: Introductionmentioning

confidence: 99%

Is there a relationship between outer retinal destruction and choroidal changes in cone dystrophy?

Ayyıldız¹,

Özge²,

Küçükevcilioğlu³

et al. 2016

Arquivos Brasileiros De Oftalmologia

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Approved by the following research ethics committee: GATA Medical School (# 2015-KAEK-45). ABSTRACT Purpose:The aim of the present study was to use enhanced depth imaging optical coherence tomography (EDI-OCT) to investigate choroidal changes in patients with cone dystrophy (CD) and to correlate these findings with clinical and electroretinography (ERG) findings. Methods: This case-control study included 40 eyes of 20 patients with CD and 40 eyes of 40 age-and refraction-matched healthy individuals. Choroidal thickness (CT) measurements were obtained under the foveal center and at 500 and 1,500 µm from the nasal and temporal regions to the center of the fovea, respectively. EDI-OCT and ERG data were analyzed, and the correlations of CT with the best-corrected visual acuity (BCVA) and the central foveal thickness (CFT ) were evaluated. Results: The mean subfoveal CTs in the CD and control groups were 240.70 ± 70.78 and 356.18 ± 48.55 µm, respectively. The subfoveal CT was significantly thinner in patients with CD than in the controls (p<0.001). The patients with CD also had significantly thinner choroids than the controls at each measurement location relative to the fovea (p<0.001). The subfoveal CT in the CD group correlated with CFT (p=0.012), but no significant correlation was found between the subfoveal CT and BCVA or photopic ERG responses. Conclusions:The present study demonstrated a significant thinning of the choroid in patients with CD. EDI-OCT is a useful technique for describing the choroidal changes occurring in CD. Future studies investigating the association between choroidal changes and outer retinal destruction or the disease stage may provide a better understanding of the pathophysiology of CD.

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Section: Introductionmentioning

confidence: 99%

Is there a relationship between outer retinal destruction and choroidal changes in cone dystrophy?

Ayyıldız¹,

Özge²,

Küçükevcilioğlu³

et al. 2016

Arquivos Brasileiros De Oftalmologia

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“…The IZ line is vulnerable to damage and is disrupted in virtually every kind of macular diseases [5] [20]. Disruption of the IZ line has been reported both in RP [12] and in CRD [19], but the difference between these retinal dystrophies is not known. The results of the current study showed that the IZ line is visible in some patients with RP, but not in any patients with CRD, even though patients had similar visual acuity.…”

Section: Discussionmentioning

confidence: 99%

“…in RP [9][10][11][12], cone dystrophy [13][14][15][16][17], and CRD [18]. In addition, a recent study suggested that the IZ line is useful in differentiating between different types of photoreceptor degenerations because cone photoreceptors are primarily impaired in CRD [19]. However, it is not known if changes of IZ line is specific to CRD because IZ appearance on OCT is sensitive to any kind of photoreceptor damage [20].…”

Section: Introductionmentioning

confidence: 99%

Tomographic comparison of cone-rod and rod-cone retinal dystrophies

Inui

Oishi

et al. 2014

Graefes Arch Clin Exp Ophthalmol

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“…A disruption or loss of the macular inner/outer segments junction of the photoreceptors was shown in achromatopsia [3,11,12]. Absence of an interdigitation zone was found in cone-rod dystrophy [13].…”

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confidence: 99%

“…Optical coherence tomography and FAF imaging in children with nystagmus is challenging due to unpredictable co-operation and unstable fixation in nystagmus [6,20,21]. The groups studied so far have usually been small and mostly included older children and adults using various OCT machines [9][10][11][12][13][22][23][24]. Similarly, the retinal thickness val-ues of healthy children in the literature applied only to a group of children studied, while generally accepted normative values for the paediatric population are not yet available [25][26][27][28][29].…”

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confidence: 99%

Optical coherence tomography and fundus autofluorescence in children with infantile nystagmus syndrome and early-onset retinal dystrophy

2019

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BaCKground: The purpose of the study was to compare morphological retinal differences in children with infantile nystagmus syndrome (INS) and early-onset retinal dystrophies (EORD) with healthy controls in order to evaluate the diagnostic value of optical coherence tomography (OCT) and fundus autofluorescence (FAF). Material and Methods: Twenty-six children (mean age 10.0 years) were ophthalmologically and electrophysiologically diagnosed as INS and EORD. Their SD-OCT and FAF imaging was prospectively performed and compared with 36 controls (mean age 7.2 years). results: Optical coherence tomography imaging was successfully completed in 17/26 (mean age 9.7 years) and FAF in 15/26 (mean age 11.1 years) children with EORD. OCT imaging was completed in 29/36 (mean age 8.9 years) and FAF in 15/19 (mean age 8.7 years) control children. In all six children with Leber congenital amaurosis (LCA) photoreceptor layer abnormalities were seen in the peripheral retina using SD-OCT, but macular images could not be obtained. In 5/5 children with congenital stationary night blindness (CSNB) retinal thinning was found in all regions except the fovea. 2/6 children with achromatopsia showed ellipsoid zone disruption with macular thinning. In 1/1 child with blue cone monochromatism reduced macular thickness was observed. In 5/5 children with cone-rod dystrophy the outer retinal signal intensity was decreased. In 2/3 children with complete CSNB, slight foveal hypoautofluorescence was indicated. 1/4 children with achromatopsia showed a central hyperautofluorescent ring. In 1/1 child with blue cone monochromatism slight foveal hypoautofluorescence was observed. 2/7 children with cone-rod dystrophy showed a central hyperautofluorescent ring, and additionally in 1/7 a macular hypoautofluorescence in one eye and an additional patch of hyperautofluorescence centrally in the other eye were found. ConClusion: The study demonstrated OCT and FAF as a non-invasive and fast diagnostic tool that defines morphological changes in early-onset retinal dystrophies in young children with nystagmus.

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Outer Retina Analysis by Optical Coherence Tomography in Cone-Rod Dystrophy Patients

Cited by 40 publications

References 15 publications

Is there a relationship between outer retinal destruction and choroidal changes in cone dystrophy?

Is there a relationship between outer retinal destruction and choroidal changes in cone dystrophy?

Tomographic comparison of cone-rod and rod-cone retinal dystrophies

Optical coherence tomography and fundus autofluorescence in children with infantile nystagmus syndrome and early-onset retinal dystrophy

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